Apocrine mixed tumors of the skin illustrate the ability of pluripotential primordial cells to differentiate in different directions within an individual tumor. Moreover, mixed tumors appear as a compendium of different folliculo-sebaceous neoplasms. We suggest that these proliferations should be interpreted as "complex neoplasms of the primary epithelial germ."
We report on the case of a male who from the start of life displayed vesicular lesions; on the trunk these were clustered and on the limbs they adopted a linear configuration. After biopsy of one such lesion, the histopathological study was compatible with a diagnosis of incontinentia pigmenti (IP). In the following months, hyperkeratotic lesions appeared which later became pigmented. The mother and other female members of the family also showed different degrees of alteration related to the same disease. The karyotype study showed the existence of 47,XXY (Klinefelter syndrome). The exceptional nature of this case is that although it is the third case reported in the literature of a male affected by incontinentia pigmenti with a previous family history, it is the only one combining this characteristic with the presence of a 47,XXY karyotype.
A 12-year-old girl had a lesion located on the upper part of the scalp that was clinically interpreted as a melanocytic nevus and corresponded histologically to a basal cell carcinoma. At age 2 years she had been diagnosed as having acute lymphoblastic leukemia L1, for which she was treated with systemic chemotherapy. She also received telecobalt therapy to the whole cranium for prophylaxis of meningeal leukosis. Having rejected other possible causes that can favor the appearance of basal cell carcinoma during childhood (basal cell nevus syndrome, nevus sebaceus, albinism, etc.), we believe that this case should be added to the five previously reported in the literature as basal cell carcinoma developing at an early age in cranial radiation portals with megavoltage radiotherapy. We draw attention to the need to explore radiated areas of skin as part of the long-term follow-up of these patients, and to the advisability of obtaining a biopsy from any persistent lesion in these areas.
A 3-year-old boy had maculopapules on his face and neck since age 6 months. These were yellow-brown, asymptomatic, and clinically similar to flat warts. Histopathologic study revealed a fibrohistiocytic infiltrate in the superficial dermis. Ultrastructurally, comma-shaped bodies, desmosome-like junctions, and coated vesicles were seen; there were no lipid droplets or Birbeck granules. With these data, a diagnosis of benign cephalic histiocytosis was made. Twenty-five cases are reported in the literature: 17 males and 8 females (male:female ratio 2:1). Sixteen patients had lesions on parts of the body other than the head, neck, and shoulders.
The title "digital mucoid cyst" is generic and encompasses cutaneous myxomas, which are focal intradermic mucinoses that are at times cystic, and ganglia, which are subcutaneous teno-arthro-synovial "hernias." A large myxoma over the second and third phalanges of the middle finger of the left hand is reported that caused progressive functional incapacity. It was managed by surgery, the technique of which is described. The need to differentiate cutaneous myxomas from ganglia is emphasized because the treatment of them is different.
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